ORIGIN A L A RTICLE
Esophageal Atresia with Tracheoesophageal Fistula: Ten Years of Experience in an Institute Chia-Feng Yang, Wen-Jue Soong*, Mei-Jy Jeng, Shu-Jen Chen, Yu-Sheng Lee, Pei-Chen Tsao, Betau Hwang, Chou-Fu Wei1, Tai-Wai Chin1, Chinsu Liu1 Department of Pediatrics, and 1Division of Pediatric Surgery, Department of Surgery, Taipei Veterans General Hospital, Taipei, Taiwan, R.O.C.
Background: Esophageal atresia (EA), tracheoesophageal fistula (TEF), or both is a complicated problem. The purpose of this study was to evaluate the outcomes and postoperative complications in patients with EA/TEF who were admitted to our hospital. Methods: In total, 15 patients were enrolled from 1994 to 2003, including 8 males and 7 females. Patient demographics, associated anomalies, and outcomes were analyzed. Results: The most common variant was EA with a distal TEF (type C), which occurred in 12 patients (80%). The latter had associated congenital anomalies, and cardiac anomalies were the most frequent, occurring in 8 patients (53.3%). Of the 6 cases who had life-threatening anomalies, 4 (66.7%) died, and of the 9 cases who had no life-threatening anomalies, 2 (22.2%) died. Tracheomalacia and/or stenosis were diagnosed in 8 patients (66.7%) postoperatively. Though 3 of the 4 cases who suffered from dying spell received intratracheal stent implantation, 2 cases still died. Conclusion: The survival rate of the patients with EA/TEF is influenced mainly by associated life-threatening anomalies. TMS combined with a history of dying spell may be the major fatal complication. [J Chin Med Assoc 2006;69(7):317–321] Key Words: congenital anomalies, esophageal atresia, intratracheal stent, tracheoesophageal fistula
Introduction Congenital esophageal atresia (EA) and tracheoesophageal fistula (TEF) are well-known congenital anomalies which affect 1 in 2,400–4,500 live births.1 The survival of infants born with both EA and TEF (EA/ TEF) has dramatically improved since Cameron Haight’s first report of successful surgical correction in 1941.2 Improvement of survival is multifactorial and largely attributable to the advances in neonatal intensive care, anesthetic management, ventilatory support, and surgical techniques over the past decades. Survival can even be achieved in low birth weight infants,3 and mortality is currently limited to those with coexistent severe life-threatening anomalies. Despite the improvement in survival, the morbidity associated with surgical repair remains high.4 In this study, we review our previous 10-year experience of
patients with EA/TEF. The results may be beneficial for further clinical management of these patients in future.
Methods Patients who had been admitted to Taipei Veterans General Hospital with a diagnosis of EA/TEF between 1994 and 2003 were enrolled. We retrospectively analyzed their medical record charts. Their anatomical classification was based on the Gross classification (type A–D, plus H-type TEF).5 These infants were also classified into the following risk groups with Waterston and associates’ method: group A, birth weight >2,500 g and well; group B, birth weight 1,800–2,500 g and well or birth weight >2,500 g but with pneumonia and other congenital
*Correspondence to: Dr Wen-Jue Soong, Department of Pediatrics,ofTaipei Veterans General Hospital, 201, Hospital, *Clayton Chi-Chang Chen M.D., Department Radiology, Taichung Veterans General Section Shih-Pai Road, Taipei Rd., 112,Taichung, Taiwan, R.O.C. No. 160,2,Sec. 3, Taichung-Kang 407, Taiwan, R.O.C. E-mail:
[email protected] Received: December 13,26, 2005 March 7, 2006
[email protected] Received: October 2005 Accepted: Accepted: February 23, 2006 ●
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anomalies; and group C, birth weight 1,800 g with severe anomalies or pneumonia. Patient demographics, associated anomalies, postoperative complications, and outcomes were studied. The follow-up period ranged from 14 months to 10 years.
Results In total, 15 patients, 8 males (53.3%) and 7 females (46.7%), were enrolled. The mean gestational age was 37 ± 3.2 weeks (range, 27–41 weeks) and the mean
birth weight was 2,491 ± 708 g (range, 770–3,825 g). There were 3 preterm infants (gestational age –3 infections per year) occurred in 6 patients (50%) and 1 received lobectomy. Gastroesophageal reflux (GER) was found in 6 patients (50%), 2 of them received antireflux procedures. Anastomotic leakage occurred in 4 patients (33.3%). Anastomotic strictures, defined as an anastomotic narrowing that required dilatations or reoperation, occurred in 2 patients (16.7%) at esophagoesophageal (EE) and esophagocolonic (EC) anastomoses. Balloon dilatation was done in 1 patient and reoperation in the other. During the follow-up period, 7 patients (46.7%) died. Four patients died after surgical repair, 2 of whom had experienced dying spell (cases 14, 15). Four of the 6 patients with life-threatening anomalies (66.7%) died. In 9 cases who had no life-threatening anomalies, only 2 (22.2%) died. The survival rate in each risk group of A, B and C were 100%, 83.3% and 14.3%, respectively.
Discussion Several studies have reported the variety of postoperative complications and outcomes in EA/TEF patients.4–8 Respiratory complications were associated with TMS (11–62%), dying spell (2.7%), and recurrent pneumonia (90%).4,7,9 Gastrointestinal complications included GER (35–95%) and esophageal stenosis (17– 40%).5,10 Others were anastomotic leakage (8.5–36%), recurrence of fistula (4–10%), and anastomotic stricture (8–37%).5–8 The association of TMS with EA/TEF has been well recognized. It is present in pathologic specimens in 75% of EA/TEF patients.7,11 In our series, it was diagnosed in 8 patients (66.7%). The rate was higher than in the earlier studies,4,7 but a bit higher than the recent study10 because dynamic flexible airway endoscopy for the routine check of the whole airway in these patients was recently used in our hospital. Thus, we could detect more dynamic changes in TMS cases, which may otherwise be missed by computed tomography study. “Dying spell” means patients with TMS getting apnea and cyanosis, especially when they feed, cry, or cough, and can be the most serious problem associated with TMS. When TMS is severe enough to induce obvious
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clinical symptoms (dyspnea, cyanosis, or recurrent pneumonia), aggressive intervention should not be delayed.12,13 Patients who have esophageal stenosis can cause more serious problem because the food bolus may stack at the proximal site of the stenosis. This bulging pouch may anteriorly compress the membranous portion of the trachea, resulting in tracheal lumen block, then inducing apnea and hypoxia. In our series, 4 cases (33.3%) were diagnosed as having dying spell. The incidence was higher compared with the other report7 as we treated the transferred cases (cases 2, 14) with recurrent TEF and anastomotic strictures who had a higher possibility of getting dying spell after reoperation, and case 15, another dying spell case, noted to have TMS before surgery, had the condition worsen after the total correction. Three of the 8 TMS cases with dying spell had received tracheal stent placement, and 2 of them died. One survived and the stent was removed successfully 1 year later. Another TMS patient with dying spell, no stent deployed, received tracheal balloon dilatations twice and had normal respiratory status in the follow-up period. Esophageal dysmotility and GER are common sequelae in patients who have undergone repair of EA/TEF. In our series, 6 cases (50%) were diagnosed with GER. The incidence was higher than some, but not all reports4,7,10 because we had more complicated transferred cases (cases 2, 7, 14) who required reoperation that might have worsened the intrinsic motor function of the esophagus. Chittmittrapap et al4 showed that GER could significantly increase the esophageal stricture rate. Two of the GER cases had strictures over the site of anastomosis. We advocate early detection and treatment of GER to reduce postoperative stricture rate. The reported incidence of recurrent TEF ranges from 3 to 12%.4–5 The pathogenesis of recurrent TEF can be an anastomotic suture leakage with further erosion through the previous repair site. All the 3 cases with recurrent TEF in our series were transferred from other hospitals after the primary surgery, and came for further surgical repair. In 1962, Waterston et al14,15 devised the first classification systems for EA/TEF. Risk factors included low birth weight, pneumonia, and associated anomalies. Our study discloses different survival rates among groups A, B and C patients (100%, 83.3% and 14.3%, respectively). We demonstrated that this classification may predict outcomes, but the number of our patients is not large enough to make a statistically significant differentiation. The management of infants with isolated EA (type A) is often complex and presents a challenge for
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pediatric surgeons. The operative morbidity associated with esophageal replacement procedure is high.5,16,17 In addition to the anastomotic leakages, strictures, and graft ischemia, the other late complications include GER, recurrent aspiration, esophageal dysmotility, swallowing problems, ulceration, bleeding, respiratory tract infections, and failure to thrive.5,17–19 One case of isolated EA in our series had received reconstruction with right colon, which was complicated with esophageal stricture, and who subsequently had balloon dilatations done 4 times, suture site leakage, recurrent pneumonia, and pneumonectomy. She is 11-years-old and has normal respiratory status without any other complications. The mortality rate in our series is higher than in other reports7,8 because of the more complicated nature of the transferred cases. We conclude that the survival rate of patients with EA/TEF was influenced mainly by associated lifethreatening anomalies and postoperative TMS with dying spell. Therefore, we suggest that EA/TEF patients with clinical respiratory distress should be evaluated aggressively by flexible airway endoscopy that can be used not only for early diagnosis but also for intervention.
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